Quality-of-life outcomes in adults and children with Chiari 1 malformation and in those managed without surgery: a prospective, multicenter, observational study

BACKGROUND AND OBJECTIVES: Chiari 1 malformation (CM1) is a common MRI finding and a frequent reason for neurosurgical consultation. Although many studies have investigated surgical outcomes for patients with CM1, outcomes for those treated without surgery have been less frequently reported. The UK Chiari 1 Study reports the quality of life of adults and children with CM1 treated without surgery, 12 months after the first neurosurgical clinic visit.

METHODS: The UK Chiari 1 Study was a prospective, multicenter cohort study of adults (≥16 years) and children (<16 years) with CM1. This was an observational study that did not alter the course of clinical care. Symptoms and quality-of-life data (using Short-Form 36 in adults and the Pediatric Quality of Life Inventory™ in children) were collected at baseline and 12 months after the first clinical review for all participants.

RESULTS: One hundred ninety-two patients with CM1 (146 females; 148 adults) were studied at baseline, and 113 patients with CM1 treated without surgery were studied at a 12-month follow-up. Baseline quality-of-life scores in the study cohort were significantly lower in every domain compared with normative control data, in both adults and children. There were no decreases in quality-of-life subscores after 12 months in this cohort of adults and children with CM1 treated without surgery. Social functioning (t = −40, P < .001) and bodily pain (t = −2.9; P = .03) Short-Form 36 scores showed improvements at 12 months in adult patients treated without surgery.

CONCLUSION: This study demonstrates the stability of quality-of-life domains in adults and children with CM1 after 12 months who have been managed without surgery. Further studies are required to understand the determinants of poor quality of life in patients with CM1 and to investigate interventions for improving quality of life. There is a further need for robust comparison of surgical and nonsurgical management for patients with CM1.